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Date of last update: 10/09/2017.

Forum Name: Renal Failure

Question: Renal Artery Stenosis-Polycystic Kidney Disease

 gibsonjohnp - Wed Aug 17, 2005 8:10 am

Can you please assist. I have a 5yr old son with Renal Artery Stenosis and Polycystic Kidney Disease (both kidneys). His blood pressures have been consistently high for the past two months (high 132/65, low 120/60). For several years he ws running 90/60. Blood levels are now showing off. Creatinine is showing at 1.2. BUN is allso showing high in blood. 24hr urine is showing low levels of Urea and Creatinine. He is currently on a low sodium, low potasium diet. Taking Enalapril HCTZ, Atenolol, and Norvasc twice a day to try and stabalize his blood pressures and it is not working. Current Renal docs can not determine why pressures keep going up. Is this Uriema? The docs are arguing back and forth about it and my child is not getting any better.
 bruin75 - Sat Sep 10, 2005 7:30 pm

Your child probably has what is called Autosomal Recessive Polycystic Kidney Disease or ARPKD. ARPKD (also called infantile polycystic kidney disease) typically presents in infancy, although there are childhood and adolescent forms which are generally less severe The estimated incidence is 1:10,000 to 1:40,000. ARPKD is frequently associated with hepatic involvement, characterized by cysts, fibrosis, and portal hypertension. Hypertension (high blood pressure) is common during the first few years, even though the plasma creatinine concentration may be normal or only slightly elevated. Progressive renal failure ultimately occurs in most patients. However, the glomerular filtration rate, that is the rate at which the kidneys can filter the blood, follows a fluctuating course. It is often decreased for the first weeks of life, then increases during the first two years. It may in fact remains stable for several years, and then gradually declines. End-stage renal disease usually occurs after 15 years of age, although onset between ages 5 and 10 may be seen in children who present in the first year of life . However, a small subset of patients continues to have autonomous renal function at 40 to 50 years of age. Unfortunately, your childs kidney's will not get better, it is an indolent and progressive disease that is a result of genetic mutations in chromosome 6. The defective gene has been isolated and has been cloned The extremely large gene, PKHD1, encodes a novel protein product, named fibrocystin also known as polyductin, that is comprised of over 4000 amino acids. Eventually he will need renal replacement therapy (dialysis) and will be a candidate for a kidney transplant.
You should be aware that each child or new fetus will have a one in four chance of developing the disease (although the expression of the disease may be different from that in other siblings) and has fifty percent chance of being a carrier of the defective gene.
Your child appears to be managed appropriately by his nephrologist. He is on the right medications for his blood pressure, particularly an angiotensin converting enzyme inhibitor. These medications reduce kidney injury by reducing the pressure inside the kidney, however, they do cause a rise in the serum creatinine but that is not a bad thing. I would have trust in your childs physicians as they sincerely have the interest of your childs care at heart. The details of his care may not always be agreed upon by each individual phyisician to which you have entrusted the care of your chiild. To the uninitiated that seems as if they don't have any idea of what to do. I can assure you that these kinds of disagreements occur frequently in medicine and in science in general. It is as a result of these disagreements that the best ultimate results are obtained in the end.

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